Special Health Care Needs Flashcards
What is the definition of special health care needs?
Any physical, developmental, mental, sensory, behavioral, cognitive or emotional impairment or limiting condition that requires medical management, healthcare intervention and/or use of specialized services or programs
How many children with SHCN under 19 years of age in the US?
10.2 million (representing 14% of all US children) and <6000 pediatric dentists Different source: 12.5 million and 18% of children in the US
How many adolescents in the US with SHCN cross into adulthood?
750,000
When should specific transition planning begin for those patients with SHCN?
Between the ages of 14-16 years
What is the most common category of unmet health care for children with special needs?
Dentistry!
T/F:According to the Americans with Disabilities Act (AwDA), the dental office is a place of public accommodation.
True. Thus, dentists are obligated to be familiar with these regulations and ensure compliance. Failure to accommodate pts with SHCN could be considered discrimination and violation of federal and/or state law! E.g. provide wheelchair ramps, disabled-parking spaces
T/F: Children with SHCN are more likely to be victims of physical abuse, sexual abuse, and neglect compared to those w/o disabilities.
True
What are the SBE prophylaxis guidelines?
- Prosthetic cardiac valve of prosthetic material used for cardiac valve repair
- Previous infective endocarditis
- CHD (congenital heart disease) - Unrepaired cyanotic CHD, including palliative shunts/conduits - Completely repaired cong heart defect with prosthetic material or device, whether placed by surgery or catheter, during first 6 months after procedure (bc endothelialization of prosthetic materials occurs within 6 months after the procedure) - Repaired CHD with residual defects at or adjacent to site of prosthetic patch or prosthetic device (which inhibit endothelialization)
- Cardiac transplant recipients who develop cardiac valvulopathy **these cardiac conditions are associated with the highest risk of adverse outcome from infective endocarditis
Does an anesthetic injection through non-infected tissue need SBE prophylaxis?
No. Also, SBE prophylaxis not required for X-raysremovable prosth or ortho appliance placement ortho appliance adjustmentsortho bracket placementexfoliation of deciduous teethbleeding from trauma to lips or oral mucosa
What are some non-cardiac factors that can place pt with compromised immunity at risk for distant-site infection from dental procedure?
- Immunosuppression HIV, SCIDS (severe combined immunod), neutropenia, cancer chemo, hematopoietic stem cell or solid organ transplantation - head and neck radiotherapy - autoimmune disease (Juvenile arthritis, systemic lupus erythematosus) - sickle cell anemia - asplenism or s/p splenectomy - chronic steroid usage - diabetes - bisphosphonate therapy
What are AHA recommendation for abx px for indwelling catheters or medical devices?
AHA recommends abx px for - nonvalvular devices indicated only at time of placement to prevent surgical site infection* - indwelling vascular catheters (central lines) - cardiovascular implantable electronic devices (CIED) - VA, VC, VV shunts do require abx px (VAC) - VP shunt does not (does not involve any vascular structures) *AHA found no convincing evidence that microorganisms associated with dental procedures cause infection of nonvalvular devices at any time after implantation – most often gram negative staph or other microorganisms non-oral in origin associated with surgical implantation or other active infections
What patients are at increased risk of hematogenous total joint infection?
- All patients with a prosthetic total joint replacement - Previous prosthetic joint infection- Inflammatory arthropathies (e.g. RA, SLE) - Megaprosthesis - Hemophilia - Malnourishment - Compromised immunity
ADA/AAOS 2012 recommendations that are endorsed by the AAPD:
- Abx px not indicated for: - pins, plates, screws, or other hardware that is not within a synovial joint - nor routinely for most dental patients with total joint replacements Based on the fact that antibiotic prophylaxis has NOT shown a significant reduction in the risk of developing joint infections subsequent to dental procedures
What are considered high-risk dental procedures?
- All procedures that involve manipulation of gingival tissue or periapical region of teeth or perforation of the oral mucosa
Oral abx dosage for abx px:
Amoxicillin: 2g adult, 50 mg/kg child Allergy to pens or ampicillin => Cephalexin: 2g adult, 50 mg/kg child Clindamycin: 600 mg adult, 20 mg/kg child Azithromycin or clarithromycin: 500 mg adult, 15 mg/kg child
ABX PX for pt unable to take oral medications:
All given IM or IV Ampicillin 2g adult, 50 mg/kg child Cefazolin or ceftriaxon 1g adult, 50 mg/kg child Allergic to penicillin or ampicillin: Cefazolin or ceftriaxon 1g adult, 50 mg/kg child Clindamycin 600 mg adult, 20 mg/kg child
Amoxicillin is the drug of choice for SBE prophylaxis because of increased A. Compliance B. GI absorption C. Sustained serum levels A&B B&C
B and C
What type of chx is indicated for pts tx w/chemo or radiation?
Alcohol-free CHX because otherwise high alcohol content may cause discomfort and dehydrate the tissues in pts with mucositis.
Is flossing and ultrasonic toothbrushing recommended for all patients undergoing cancer therapy?
No, only for those that are properly trained. Brushing of teeth and tongue 2x day with soft brush or electric toothbrush is recommended regardless of hematological status If poor OH and/or perio disease, may use CHX rinses daily until tissue health improves or mucositis develops.
What are the important ANC values?
> 2000 : no need for abx px 1000 – 2000 : rx abx px (consider more abx if infection/unclear) < 1000 : defer elective dental care. In emergency, discuss abx coverage with medical team before proceeding with treatment. Pt may need hospitalization for dental management. <500 defer all care
What are important platelet counts?
> 75,000 doing awesome, no additional support needed 40k – 75k : consider platelet transfusion pre- and 24 hrs post-operatively. Use localized control measures like: sutures, hemostatic agents, pressure packs, gelatin foams < 40k : defer care. In emergency, discuss w/PMD platelet transfusions, bleeding control, hospital admission. Also use localized control like: microfibrillar collagen, topical thrombin and additional meds recommended by hematologist/oncologist like aminocaproic acid, tranexamic acid
How do cycles of chemo (and other therapies) typically affect blood counts?
Blood counts start falling 5-7 days after beginning of cycle. Stay low for 14-21 days Rise to normal a few days before next cycle begins. Ideally all dental tx complete before therapy begins. Otherwise, place ITR and delay non-acute dental tx.
How are dental procedures prioritized for pts undergoing cancer therapy?
FIRST: 1. Infections 2. Extractions 3. Periodontal care (scaling, px) 4. sources of tissue irritation THEN: 5. carious teeth (look at risk for pulp infection & pain) 6. RCT permanent tth 7. replacement faulty restorations Be aware that s/s periodontal disease may be decreased in immunosuppressed pts.
What is the recommendation for pulp therapy for primary teeth prior to cancer tx?
NO STUDIES showing safety. Definitive tx (EXT) preferred by clinicians Pulpal/PA/furcal infections during immunosuppression periods can become life-threatening Existing pulp/sscs should be monitored for signs internal resorption/failure to do infection
Recommendation for permanent tooth endo tx prior to cancer tx?
Symptomatic, non-vital permanent tth: do RCT > 1 wk before chemo bc need time to assess success of RCT Asymptomatic, non-vital perm tth RCT may be delayed until hematological status of pt is stable. Otherwise, EXT EXT followed by abx for 1 wk if RCT can’t be completed in single visit **determine etiology of PARL associated with RCT-txed teeth to diagnose pulpal infection, inflammatory rxn, apical scar, cyst, malignancy. If PARL associated with RCT tooth & no s/s infection, could be apical scar (no need for retreat or ext)
Recommendations for ortho/space maintainers during chemotherapy:
If OH poor, tx protocol or HCT conditioning has moderate to high risk of developing mucositis, remove appliances. If can’t remove, use wax/mouth guard to decrease tissue trauma If poorly fitting, remove bc can abrade mucosa and increase risk of microbial invasion of deeper tissues Simple things like B&L, LLHA that are not irritating may be left in place for pts with good OH. Removable appliances may be worn as tolerated. Clean them with antimicrobial solutions
Perio considerations for chemo therapies
Operculum should be excised if potential risk for infection d/t pericoronitis & if hematologic status allows. Perio assessment needed prior to bisphosphonate therapy part of cancer tx EXT for tth with poor prognosis that can’t be treated definitively w/perio therapy
What should be done with loose primary teeth during cancer tx?
Allow them to exfoliate naturally
Nonrestorable tth Root tips Tth with > 6mm perio pockets Symptomatic impacted teeth Teeth w/acute infections should be extracted ideally how far before therapy starts?
Ideally 2 weeks At least 7-10 days allow adequate healing
Do pediatric dentists encourage foam or super soft brushes?
No, we discourage them because they do not allow for effective cleaning – only use if pt develops moderate to severe mucositis Toothpicks and water irrigation devices should not be used when pt is pancytopenic (avoid tissue trauma)
Are lanolin or petroleum based products more effective for lip care?
Lanolin
Oral complications related to cancer therapies: 54%
mucositis salivary effects- reduced flow, thicker, lower pH opportunistic infx (fungal, viral, bacterial) oral bleeding: thrombocytopenia, others soft tissue necrosis dental sensitivity/pain: salivary changes, neurotoxic drugs, odontogenic infx, referred pain taste dysfunction mucosal and muscular fibrosis trismus, TMJ dysfunction nutritional deficiencies GVHD in HSCT craniofacial, dental development problems
Management of mucositis includes medicated mouth rinses
No. Recommend good OH, analgesics, non-medicated rinses like 0.9% saline or sodium bicarb rinse 4-6xday, parenteral nutrition as needed
What is palifermin and what is it recommended for?
Palifermin is keratinocyte growth factor-1Recently recommended for prevention of oral mucositis as prophylaxis and tx to decrease mucositis Observed to decrease incidence and duration of severe oral mucositis in pts undergoing conditioning w/high dose chemo with or without radiotherapy, followed by HCT (hematopoietic cell transplant)
What else is recommended for mucositis?
Coating agents like Amphojel Kaopectate hydroxypropylmethylcellulose Film-forming agents like Zilactin Gelclair low-level laser therapy – limited but encouraging evidence NOT RECOMMENDED: sucralfate, antimicrobial lozenges, pentoxifylline, and granulocyte-macrophage-colony stimulating factor mouthwash chlorhexidine not recommended for prevention of mucositis
What do “Philadelphia mouthwash” and “Magic Mouthwash” have?
Topical anesthetics. No significant evidence of the effectiveness or tolerability of these. Only provide short term pain relief. Lidocaine use may obtund or diminish taste and the gag reflex and/or result in a burning sensation, in addition to the possible cardiovascular and CNS effects
T/F: Prophylactic nystatin is effective for the prevention and/or treatment of fungal infections in cancer pts.
False
What dental side effects can vincristine or vinblastine (plant alkaloid chemo) have?
May present w/ deep, constant pain affecting mand molars in absence of odontogenic pathology. Pain usually transient & subsides shortly after dose reduction/cessation of chemo
Are saliva stimulating drugs approved for use in children?
No. Recommended: sugar-free chewing gum or candy, sucking tablets, special dentifrices for oral dryness, saliva substitutes, frequent water sipping, alcohol-free oral rinses, oral moisturizers, humidifier, fluoride rinses and gels
When can you start ortho on a child who has undergone cancer therapy?
After completion of all therapy and after at least a 2 year disease-free survival, when the risk of relapse is decreased and pt is no longer on immunosuppressive drugs
What are the 5 phases of Hematopoietic cell transplant?
Phase I: Preconditioning Phase II: Conditioning neutropenic phase Phase III: Engraftment to hematopoietic recovery Phase IV: Immune reconstitution/recovery from systemic toxicity Phase V: Long-term survival
How does HCT differ from other cancer therapies?
- HCT pt receives all the chemo and/or total body irradiation in just a few days before the transplant2. There will be prolonged immunosuppression following transplant.
Until when must elective dentistry be postponed after HCT?
Until immunologic recovery At least 100 days after HCT Longer if GVHD or other complications present *tx should be completed before pt becomes immunocompromised
Phase II: Conditioning neutropenic phase starts when and lasts how long?
Starts when pt admitted to hospital to begin transplant conditioning until 30 days post HCT - mucositis (begins 7-10 days after initiation & last abt 2 wks after end of conditioning) - xerostomia - oral pain - hemorrhage - opportunistic infections - taste dysfunction - neurotoxicity (including dental pain, muscle tremors) - TMD (pain, HA, joint pain) - GVHD (with allogeneic transplant) – severe inflammation/ mucositis *Dental tx usually not allowed in this phase due to severe immunosuppression
Phase III: Engraftment to hematopoietic recovery has what concerns? Lasts 1 month to 100 days
Complication severity decrease 3-4 wks after transplant Oral fungal and herpes simplex infections notable Acute GVHD xerostomia, hemorrhage, neurotox, TMD, granulomas/papillomas sometimes are observed Acute sensitivity to thermal stimuli btwn 2-4 months s/p tx w/spontaneous resolution w/in few months. Use topical F- to reduce symptoms.
Phase IV: Immune reconstitution/recovery from systemic toxicity starts when and involves what?
After day 100 s/p HCT Oral complications related to chronic toxicity: - salivary dysfunction (+xerostomia) - craniofacial growth abnormalities - late viral infections - oral chronic GVHD - oral squamous cell carcinoma Mucosal bacterial infections less frequently seen, unless pt is neutropenic or with severe chronic GVHD Exam + xrays can be done. Defer invasive dental tx in profoundly immunocompromised pts.
Phase V: Long-term survival Craniofacial, skeletal, and dental development complications occur for children under what age?
< 6yo at time of cancer therapy (<4 y.o. especially per Flaitz) - tooth agenesis - microdontia - crown disturbances (size, shape, enamel hypoplasia, pulp chamber anomalies) - root disturbances (early apical closure, blunting, changes in shape or length) - ectopic eruption - reduced mandibular length - reduced alveolar process height - malocclusion, facial deformities, skeletal hypoplasia - trismus - may experience permanent salivary gland hypofunction/dysfunction or xerostomia - increased caries risk Relapse can develop during stage V
When are dental malformations most severe for children undergoing chemo/radiation therapy?
< age 6, Especially < 4yo - tooth agenesis - crown malformation/microdontia - enamel hypoplasia - root V shape, blunting, altered root number
What are the 2 types of Hematopoietic Stem Cell Transplantation? (HSCT)
- BMT 2. PBSCT Peripheral blood stem cell transplantation Purpose: to restore stem cells that are destroyed by doses of chemo or radiation therapy
What are the 3 types of transplants?
- Autologous: pt’s own stem cells 2. Syngeneic: stems from identical twin 3. Allogenic: stems from HLA-compatible donor, related or non-related
What are the two types of cancer most frequently treated with HSCT?
Leukemia + lymphoma Also, aplastic anemia, solid tumors like neuroblastoma, inborn and acquired immune disorders
What are the 4 phases of HSCT care?
- Pretransplantation phase 2. Conditioning/ neutropenic phase 3. Initial engraftment to hematopoietic reconstitution 4. Immune reconstitution/late post-transplantation
What period lasts from day 0 to 30 days post-HSCT?
Conditioning / neutropenic Acute GVHD begins at this time
When do complications from HSCT begin to decrease?
4 wks after transplantation GVHD may be present sensitivity to thermal stimuli
When does GVHD develop?
Allogenic transplant Transplanted T-lymphocytes recognize host’s cells as foreign Acute: occurs up to day 120 post-transplant - rash, GI, hepatitis - mucosal atrophy/erythema/lichenoid rxn tx: steroids/immunosuppressants Chronic GVHD: occurs btwn 70-500 days post-transplant (avg 6 months) - 20-50% of children (higher in adults) - immune dysfn or autoimmune - many organs affected: skin, liver, joints, eyes, mouth, lung, muscle - in addition to atrophy/erythema/lichenoid/xerostomia/ulcers/gingivitis/caries/perio/trismus/taste dysfunction - cyclosporine => gingival overgrowth
Herpangina is caused by what virus?
Coxsackie A (enterovirus) also causes hand food mouth dx 2-6 red macules, vesicles, ulcers on the soft palate, tonsillar pillars; ulcers heal in 7-10 days sore throat, dysphagia, fever, rhinorrhea, cough, vomiting, headache, diarrhea
DiGeorge syndrome is what chromosomal anomaly?
Deletion of 22q11.2
DiGeorge is also called what?
Velocardiofacial syndrome VCFS
What is a good way to remember DiGeorge?
CATCH – 22 C – cardiac A – abnormal facies T – thymic hypoplasia C – cleft H – hypocalcemia 22 – chromosome 22q11.2
C- cardiac anomalies seen in DiGeorge/VCFS
- VSD - truncus arteriosus (one vessel instead of two leaving heart)- Tetralogy of Fallot
What four malformations of the heart does Tetralogy of Fallot exhibit?
- Pulmonary stenosis (narrowing of R V outflow) 2. Overriding aorta (aorta situated above the VSD and connected to both R and L Ventricle) 3. VSD (hole btwn R & L V) 4. RV Hypertrophy (R ventricle extra muscular bc of obstruction to outflow)
A – Abnormal facies of DiGeorge
Small, low set ears wide set eyes hooded eyes long face flattened philtrum
T – thymic hypoplasia results in what? (DiGeorge)
T-cells mature in the thymus. Children with small or missing thymus have poor immune function and frequent, severe infections Thymus decreases in size & function with age.
C - clefting (DiGeorge)
Cleft palate is very common (overt or submucous cleft) May cause Speech problems VPI
DiGeorge hypocalcemia results from what?
Hypoparathyroidism Parathyroid glands regulate PTH if less PTH then you have low levels of Ca and high levels of Phosphorus in the blood
Dental findings in Hypothyroidism (cretinism)
Delayed tooth eruption but normal development; +/- enamel hypoplasia; enlarged tongue, lips (glycosaminoglycans) also FTT, short, generalized edema, puffy face, husky voice, intellectual disability mandible underdeveloped, maxilla overdeveloped
When does cleft palate/lip repair occur?
Lip – at 3 months Palate – at 10-12 months because negatively impacts speech development if done later
With what frequency does clefting occur?
1:500 – 1:1000 Prevalences (AAPD COMP REVIEW) 1/2000 cleft palate alone 1/1000 cleft lip alone CL+CP 45%, CP alone 30%, CL alone 25% genetic, environmental causes (maternal alcohol use, cig smoking, folic acid deficiency, meds)
At what point developmentally does clefting occur?
4-7th week of gestation
Does cleft lip have a gender predilection?
Boys :girls, 2:1
Does cleft lip occur on a particular side with greater frequency?
Left side more frequency
Does cleft palate have a gender predilection?
Cleft palate more common in girls
By what week in utero are the palatal shelves fused?
11th week
What % of kids with asthma are allergic to NSAIDS?
4%
How long does mast cells to degranulate and lead to bronchoconstriction?
30 minutes
What are the late phase and ultra-late phase of an asthma attack?
Late (8-12 hrs later) Airway inflammation Ultra-late phase (days to weeks later) Bronchial hyper-responsiveness
What classifies mild asthma?
Mild intermittent/mild persistent - no night symptoms - tolerates exercise - wheezing <2 days/wk
What classifies moderate asthma?
- Some night systems - Limited tolerance to exercise - Wheezing 2-5 days/wk
What classifies severe asthma?
- Frequent night symptoms - Poor tolerance to exercise - wheezing every day
What are some short-acting bronchodilators?
Albuterol B2 agonist (relaxes bronchial sm muscle), rescue inhaler Proventil, Ventolin Ipratroprium Atrovent – anticholinergic, blocks Ach receptors on sm muscle thus relaxes sm muscle
What are some long-term control meds for asthma?
corticosteroids inhaled anti-inflammatory and immunosuppressive Fluticasone (Flovent), Budesamide (Pulmicort) Prednisolone (systemic)
What is a Long acting B2 agonist (12 hrs bronchodilation)?
Salmeterol, formoterolused In combo with inhaled corticosteroids (when those aren’t controlling asthma by themselves), i.e. Advair = salmeterol + fluticasone Symbicort = formoterol + budesonide
What is LTRA – leukotriene receptor agonist?
Block leukotrienes (released by cells, responsible for asthma symptoms) Singulair (Montelukast), Accolate used for seasonal allergies and maintenance tx of asthma
What role does an anti-IgE agent Omalizuab Xolair play in controlling asthma?
Used for severe persistent asthmaRecombinant DNA IgG that binds to free IgE in blood
What drugs should you use and avoid in child with mild-moderate asthma?
N2O – appropriate AVOID narcotics, barbiturates Can use Vistaril/Atarax (hydroxyzine) antihistamine Be prepared to rescue the patient.
Management of acute asthma attack:
- Discontinue tx 2. Remove everything from mouth 3. Position pt for comfort 4. Pulse-OX 5. Inhaler B2 6. Oxygen If spO2 < 91% and struggling to breathe, Epinephrine 0.01 mg/kgcall 911
Asthma is more common in Afr Am and urban dwellers. T/F
Yes. 13.4% Afr Am, 7% urban more common in low SES Childhood prevalence is 8.5% Most common chronic medical condition of childhood.
Differential Dx of asthma includes?
- Congenital abnormality of airway - Cystic fibrosis - Immunodeficiency - GERD - Foreign body - Malignancy
Albuterol is what type of drug?
Beta2-agonist
What two classes of sedation drugs should be avoided with asthmatics?
- Barbiturates (phenobarbital) 2. Narcotics (morphine & meperidine(=demerol)) stimulate histamine release leading to bronchospasm
What drugs are recommended for sedation of child w/asthma?
- Hydroxyzine (vistaril, antihistamine) 2. Benzodiazepines (potentiate GABA’s effects, diazepam (valium), lorazepam (ativan), midazolam (versed))
T/F: Asthmatic patients tend to have increased OJ and greater incidence of posterior crossbite.
True. Also high palate and increased anterior facial height As well as…increased caries, candidiasis, erosion, decreased salivary flow, increased calculus & gingivitis
What are some cardiac defects with L to R shunts. Does this cause hypoxia?
No, but can appear as congestive heart failure over time. ASD VSD PDA AVSD Increase in pulmonary blood flow at expense of systemic circulation
What is the big R to L shunt. Does it cause hypoxemia?
Yes. Less blood to lungs, pts appear cyanotic. Tetralogy of Fallot Highest risk of endocarditis
What are some obstructive cardiac defects?
Anatomic narrowing: coarctation of aorta, aortic stenosis, pulmonic stenosis valvular, subvalvular, supravalvular Also appears as congestive <3 failure over time
What are two cases of primary pump failure?
- Dilated cardiomyopathy (limited ability of <3 to contract due to abnormality of myocardium) could be from disease, medication 2. Hypertrophic cardiomyopathy – dysrhythmias, syncope, sudden death
What is congestive heart failure?
Inability of heart to pump adequate amt of blood to systemic circulation at normal pressure to meet metabolic demands Managed medically, surgically, or combination: - remove extra fluid - improve cardiac function - improve tissue oxygenation - reduce demands on heart
What are 4 medications that help manage congestive heart failure?
- Digoxin: increase contractility of <3 2. Diuretics: decrease fluids 3. Warfarin: increases prothrombin time 4. ACE inhibitors: decrease blood pressure
At around what age is an asymptomatic ASD/VSD repaired?
Age 2-4 Repaired earlier if symptomatic. Some VSDs heal spontaneously
What is the incidence of Infective Endocarditis? (IE)
5 cases / 100,000 persons-years Less frequent in children
What are the symptoms of IE?
May appear as other infection - weakness, fatigue, rash, fever, anorexia, night sweats, arthralgia - 7–14 day typical incubation period, but may take up to 4 wks *pedo patients more likely to acquire subacute rather than acute bacterial endocarditis
What are the risks of transient bacteremia from EXT, Perio surgery, SRP, cleaning, RD placement, routine daily procedures?
EXT 10-100% Perio sx 36-88% SRP 8-80% cleaning 40% RD placement 9-32% Toothbrushing/flossing 20-68% Toothpicks 20-40% CHEWING FOOD 7-51%
What do you do if you inadvertently miss the abx px?
Give dose within 2 hrs
If pt is already on abx, what should you do?
- Delay dental procedure for 10 days to allow normal flora to re-establish 2. OR choose abx from another class (azithromycin or clindamycin)
Describe primary and secondary process of hemostasis: 1. Platelet aggregation 2. Coagulation cascade
- Primary – platelet aggregation - aggregation caused by injury - release vWF & collagen fibers from endothelium - platelets adhere - vasoconstriction occurs 2. Secondary - coagulation cascade - Extrinsic pathway activated when injury exposes blood to TF - Intrinsic pathway produces F X - Common pathway generates thrombin
What are the 3 phases of coagulation?
Initiation Amplification Propagation
Describe the 3 steps of initiation:
Initiation: 1. TF binds VII 2. TF-VII activates IX + X 3. Xa + Va to produce small amt of thrombin
Describe the 3 steps of Amplification:
Amplification – occurs mainly in platelets 1. Uses thrombin from initiation activates V, VIII, IX (5, 8, 9) 2. Releases VIII from vWF 3. Ends with Va and VIIIa being bound to activated platelets (5a, 8a)
Describe Propagation in 4 steps:
Propagation: 1. Activated VIII + IX form complex in platelet 2. This complex VIII-IX activates X 3. Xa binds with Va to form prothrombinase complex 4. Prothrombinase complex converts prothrombin to thrombin
How does fibrinolysis happen?
Plasmin dissolves fibrin clotRegulated by antiplasmin and plasminogen activator inhibitors
What does warfarin inhibit?
Production of Vit-K dependent factors (II=prothrombin, VII, IX, X) effects can be reversed by administering fresh Vit K1 Onset and duration of action of single dose is 2-5 days
How does Pradaxa act as an anticoagulant med?
Pradaxa is a competitive, direct thrombin inhibitor - prevents clot development - prevents conversion of fibrinogen to fibrin
What are 5 screening lab tests for bleeding?
- PT – EXTRINSIC – tests for defects in V, VII, X, thrombin, fibrinogen (normal is 1-18 seconds ) 2. PTT – INTRINSIC – tests for defects in V, VIII, X, XI, thrombin, fibrinogen 3. Platelet count (normal = 140,000 – 340,000) 4. Platelet function (PFA-100) – more accurate than bleeding time 5. Bleeding time – inaccurate in young children (1-6 min)
Name 2 low, 4 intermediate, and 3 high risk for bleeding dental procedures.
LOW 1. supragingival restorations or px 2. infiltration anesthesia INTERMEDIATE 1. Sub-g restoration 2. single EXT 3. RCT 4. Nerve blocks HIGH 1. Multiple EXTs 2. Perio sx 3. Gingival curettage
What is the most common inherited coagulation disorder?
Von Willebrand disease 1% of populationdeficient or defective vWF (= plasma protein in endothelium) AUTOSOMAL DOMINANT INHERITANCE low levels of VIII (may have longer PTT) vW binds to VIII to protect it in blood prolonged bleeding time abnormal platelet function test VWD is a primary process defect (defect in platelet #/function) longer bleeding time, bleeding from superficial and deep cuts, petechiae, small multiple ecchymoses, spontaneous bleeding
2 ways to manage vW disease?
DDAVP (for mild Hem A or vWD) * synthetic analog of desmopressin. POTENT ANTIDIURETIC. Decrease fluid replacement rate. - causes rapid release of VIII & vWF (doubles levels) - peak effect 1 hr after administration, 30-45 min to take effect - IV or subQ - Stimate nasal spray (can repeat q24hrs, limit fluid intake 12-18 hrs) Amicar (aminocaproic acid) (prevent clot dissolution) - antifibrinolytic – inhibits plasminogen to plasmin - 2 hrs peak effect - IV or PO (50 mg/kg q6h until healed, 7 days) - may be given alone or with DDAVP - inhibits salivary enzyme fibrinolysis
Thrombocytopenia is an insufficient # of what?
Platelets Could be due to low production (aplastic anemia, BM cancer) increased breakdown of platelets (Idiopathic thrombocytopenic purpura or drug-induced immune thrombocytopenia) Symptoms: bruising, epistaxis, gingival bleeding, petechiae CBC – low platelets PTT, PT normal another primary process defect like VWD
What are the two most common Hemophilias?
Hemophilia A – 85% - Factor VIII (think ATE/EIGHT) Hemophilia B – 15% - Factor IV (think benign) Christmas 1/3 of cases are new mutations X-linked recessive inheritance secondary process defect (defect in coagulation pathway)
What define the classifications of severity for Hemophilia A and B?
Hemophilia A < 1% VIII activity severe (70%) 1-5% VIII activity moderate (15%) > 5% VIII activity mild (15%) Hemophilia B < 1% IX activity severe (50%) 1-5% IX activity moderate (30%) > 5% IX activity mild (20%)
What are the characteristics of bleeding with hemophilia?
Normal platelet plug formation (no significant bleeding after superficial cuts, but significant bleeding after deep cuts; no petechiae, but large wide-spread ecchymoses; hematoma, hemathrosis, spontaneous bleeding)delayed formation of fibrin clot (friable/poorly formed clot) Types of bleeding: Deep bleeding into joints, muscles Increased bleeding from open wounds appears to stop, then restarts (problems with exfoliation of primary teeth)
What are 5 complications of hemophilia?
- Inhibitors – Ab block clotting factor activity (15% of severe hem A, 2.5% hem B pts) (tx w/high dose clotting factor, bypassing agents, induce immune tolerance) 2. Arthropathy - can occur without injury, chronic/acute pain, joint degeneration (knees, elbows, ankles most affected) 3. Bleeding into CNS or airway 4. HIV 5. Hepatitis
What are some local measures for hemorrhage control?
- Pressure - Sutures - Collagen sponge (increases platelet aggregation) - gelfoam (absorbs blood, forms matrix to organize clot) - Surgicel (resorbable oxidized cellulose) - bone wax (mechanical barrier to bleeding from bone) - Thrombin – converts fibrinogen to fibrin - electrocautery - epinephrine (re-bleeding likely to occur)
Thrombotic disorders can be acquired or inherited. Acquired thrombotic disorders are short-duration T/F
T. T. Acquired: pregnancy, surgery, immobilization Inherited: impaired function of protein C-anticoagulant system - Factor V Leiden : 5% in North America (increased prothrombin) Management: Acute: Heparin for several days, then Warfarin for 3-6 months Long-term: Anti-coagulant therapy – verify INR
What is the normal INR? What is the therapeutic range for INR for someone on anti-coagulant therapy? What does INR measure?
Normal INR = 1 Therapeutic range 2 – 3 INR measures PT – Extrinsic pathway (F VII-TF → F X) heparin intrinsic // extrinsic warfarin
What is the target range for INR?
2-3
Sickle Cell Disease has what inheritance pattern? What is incidence of SC Trait vs Anemia/Disease in Afr Ams
Autosomal recessive Most common genetic blood disorder 1 of 12 Afr Ams have SC Trait 1 of 600 Afr Ams have SC Anemia
Sickle Cell Disease is considered what ASA?
ASA III Life expectancy ~40 yrs
What can trigger hypoxia/crisis?
- Systemic disease - Dehydration - Exposure to cold - Rigorous exercise - Infection - Acidosis - Stress
How does SC Anemia cause anemia?
Shortened life span of sickled RBC’s 12 days Normal span 120 days
What are common oral findings for pts with SC Anemia?
- Pale mucosa - Enamel hypoplasia - Dental or jaw pain from infarcts - increased incidence of osteomyelitis - delayed eruption - pulp calcification - “hair on end” lateral skull film - Class II - decreased trabeculation, thin inferior border, distinct radioopacities of mandible - decreased caries when taking abx “hair on end” also seen in neuroblastoma, Fe deficiency anemia, thalessemia
Dental considerations for SCA
- AM appts, short - No contraindications to LA w/epi - N2O use okay but minimum 50% O2 - Acetaminophen for pain - ASA III - GA ➔ hydration, oxygenation, vascular stability, temp, avoid acidosis - treat infections vigorously – avoid elective surgery
Factor VIII, when given to patients with Hemophilia A -Achieves a therapeutic level minutes after administration -Can provide enough coverage for patients with inhibitors -Has a half life of 4 hours -Is given intranasally -Should not be used in conjunction with desmopressin
Answer: a. achieves therapeutic levels minutes after adminNot always enough coverage for pts with inhibitors, half life of 8-12 hours, given IV, used in conjunction with desmopressin
What is NOT true regarding desmopressin? -Duration is 4 hours -Increases level of von Willebrand factor -Increases levels of Factor IX -Peak effect is in 20-60 minutes -Preferred over FFP for mild-moderate hemophiliacs
Answer: C. Increases levels of factor IX desmopressin (DDAVP) causes a rise in Factor VIII and vWF
Seizure disorders usually have an identifiable etiology. T/F
F. New terminology: Partial – simple or complex (40%) Generalized – convulsive or nonconvulsive (40%) Unclassified Status epilepticus – seizure lasting > 30 mins
With simple partial seizures the pt remains conscious. T/F
T. Originates from localized area of brainlocalized twitching, numbness/tinglingflashes of light, dissociation feelinglip smacking
Partial Complex seizures may be preceded by an aura. The person does not lose consciousness. T/F?
T – partial – complex – localized area of brain F – 1-2 minute loss of consciousness experienced Prevalence of seizure types: Complex partial 36% gen. tonic clonic 23% simple partial 14%
Is an absence seizure a partial or generalized seizure?
Generalized. Formerly known as petit malInvolves entire brain, 10-30 sec LOChalf develop tonic-clonic seizures at puberty
Tonic Clonic seizures were formerly known as ________.
“grand Mal” aura LOC leads to falling Tonic: muscle rigidity 10-20 secs Clonic: muscle contractions 2-5 minutes incontinence postictal period (deep sleep, HA, confusion) full recovery (3 hrs)
Phenobarbital, phenytoin, dilantin, valproic acid, depakote, Topamax, tegretol, and carbamazepine are just some examples of meds that treat what?
Seizure disorders Management of seizure disorders also includes ketogenic diet surgery vagal nerve stimulators
When do you activate EMS for a patient having a seizure?
Seizure > 5 minutes Or another seizure develops quickly since the last one status epilepticus
What is the genetic predisposition to ADHD?
40% have parent with ADHD 35% have sibling with ADHD
What are the diagnostic requirements for ADHD?
- Occur at home and school - Be more severe than in other children same age - start before age 7 - continue for >6 mo - difficult function in various settings More common in boys incidence: 3-5% school-age kids (more recent numbers 11%) Most commonly diagnosed behavioral disorder in childhood
3 common behaviors and symptoms
- Inattentive - Hyperactivity - Impulsivity Can have various combinations of these 3 characteristics
What is Oppositional defiant disorder / conduct disorder a 35% comorbidity with?
ADHD – defiance In addition, depression (18%) and anxiety disorders (25%)
Methylphenidate Ritalin and Concerta are what type of med?
Non-amphetamine / CNS stimulant ADHD med
Adderall is an amphetamine?
Yes. AKA dextro-amphetamine xerostomia, altered taste, bruxism *Caution with Meperidine!*
Strattera and Clonidine Catapres also tx ADHD
T. All ADHD meds have xerostomia as a side effect. This increases caries risk in 11-13 year olds Strattera – SNRI elevates BP – caution with vasoconstrictors Clonidine and Guanfacine (Intuniv) – anti-hypertensives also causes dysphagia potentiates CNS depressants – caution with sedative agents
T/F: Children with ADHD are at increased risk of dental caries.
True. Also increased bruxism and increased risk of dental trauma.
What is the most common form of CP?
Spastic diplegia and quadriplegia (70-80%) stiff or rigid muscles, contractures and lack of control Other types include: Hemiparesis, athetoid or ataxic, hypotonic50% CP pts have mental developmental delay, 30-50% seizure disorder, 35% sensory deficits Oral findings: increased DMFS permanent teeth lower salivary rate, lower pH → increased caries enamel erosion poor gingival health delayed eruption malocclusion- AOB, class II tongue thrust bruxism increased gag reflex dysphagia, increased drooling, mouth breathing increased trauma
IS there a gender predilection for Autism Spectrum Disorder?
Males 3-4 times more likely to be affected. 6/1000 live births for ASD (New: 1/68)
You expect to see babbling and gesturing by 12 months for a child with Autism Spectrum Disorder.
No. Also, no words by 16 months, no smiling, eye contact, lack of imagination in play, compulsive behaviors, restricted interests and activities
Applied Behavior Analysis is what type of training?
Operant conditioning to teach specific skills
What type of behaviors are targeted with meds for pts with ASD?
Hyperactivity – ritalin, concerta – enhance attention Repetitive = Prozac (fluoxetine) – antidepressants decrease compulsive beh & self-mutilation Aggressive – Tegretol (carbamazepine), Risperidone, Aripirozole (Abilify) – anti-aggression these have weight gain and sedation as side effects
What are 5 independent variables to help predict uncooperative behavior?
- Age (<4, 4-7) 2. Reading (no vs. yes) 3. Toilet trained? 4. Concurrent medical diagnosis? 5. Expressive language used? >2 of above risk factors strongly associated with uncooperative behavior
Dental/Craniofacial features of pt with Down Syndrome
— Brachycephalic and flat occiput — Midface hypoplasia — Flat bridge of nose — Alantoaxial instability — Small ears — Inner epicanthal folds — Up-slanting palpebral fissures — Relative macroglossia, fissured tongue +/- benign migratory glossitis — Open mouth facies and protruding tongue — Constricted palate — Dry, fissured lips — Crowded Class III orthognathic tendency — Microdontia — Hypodontia — Delayed eruption and over-retained teeth — Enamel hypoplasia — Decreased parotid gland salivary flow — Increased risk for periodontal disease — Increased risk for obstructive sleep apnea (30-100%)† — Increased risk for Riga-Fede disease
Down syndrome characteristic physical features
Hypotonia intellectual disability increased incidence of cardiac defects (atrioventricular septal defect most common cardiac anomaly), hypothyroidism, ALL/AML, dementia, GI anomalies, Celiac diseaserisk of infx due to immunodeficiency palmoplantar keratosis, xerosis, short broad hands; single palmar crease (simian) A-A instability, bradycardia, airway challenges, and tendency for obesity are all factors to consider with sedation or GA
What is the prevalence of periodontal disease in Down Syndrome pts <30 yrs?
50-90% 36% of pts have primary dentition involved - not a lot of calc - bacteria same as other pts w/ intellectual disability - perio pathogens colonize earlier - abnormal capillary fragility - PMNs with chemotactic/phagocytic defects- normal # B-cells - T-cells dysfunctional – may have diminished ability to recognize and respond to specific antigens
Cystic Fibrosis inheritance pattern
Autosomal recessive chronic pulmonary dx, pancreatic insufficiency, high sweat electrolyte concentrations; chronic progressive dx life expectancy: median 37 years w/ treatment, 80% reach adulthood Most common in white people (1:3200) 2-4% of whites are carriers
What is the CFTR protein?
Cystic Fibrosis Transmembrane Conductance Regulator – decreased secretion of Cl- – increased absorption of Na - exocrine glands produce thick sticky secretions → abnormal mucus formation affects lungs, intestine, upper airway and reproductive epithelium S/S: salty tasting skin failure to gain weight, abnormal bowel movementsrecurrent wheezing, cough, pneumonia; nasal polyps, sinusitis clubbing of fingers and toes pancreatic insufficiency
What are some respiratory tx for CF?
- Airway clearance techniques like chest physiotherapy, mechanical vest - exercise loosens mucus - aerosols (bronchodilators, mucolytics, decongestants, abx) - oral antibiotics
Digestive system and CF
Pancreatic enzymes blocked in duct 90% of patients affected Malabsorption of fat and protein - poor weight gain - nutritional deficiencies - high fat stools - excess gas/abdominal pain Tx: increase caloric intake, pancreatic enzyme supplements, iron, Vit A, D, E, K (fat soluble)
T/F:CF patients have higher incidence of caries and decreased calculus.
Both are False. Increased enamel defects, erosion decreased caries salivary pH elevated partial xerostomia, parotid enlargement gingivitis and increased calculus mouth-breathing, malocclusion sinusitis, rhinitis – halitosis, referred pain decreased bone density (bisphosphonates) candidiasis, angular cheilitis (chronic antibx and steroids), traumatic lesions from coughing and devices
T/F Early morning appts are recommended for pts with CF
No. bc mucous accumulation overnight and increased coughing in morning. Short appt, later in the day, more upright chair position - Beware any sedative drugs that decrease respiration - chronic abx use may increase # opportunistic pathogens in airway – consult if oral infection - chronic steroid use – increase risk for candidal infection & delayed healing
Characteristic findings of Ectodermal Dysplasia: Hair/eccrine/dental/nails
Hair – sparse, fewer follicles, rough Eccrine – poor sweat glands (heat intolerance & hyperpyrexia with 30% mortality rate, can lead to seizures/neuro) Hypoplasia of salivary (xerostomia), lacrimal glands, mucus glands of UR/GI tracts – dry skin, eyes, periorbital wrinkling and pigmentation, photophobia Dental – hypodontia to anodontia, microdontia (conical teeth), midface hypoplasia, under-developed alveolar ridges, decreased VDO Nail dystrophy (thin, brittle, deformed) Normal intelligence Ectodermal dysplasia is X-linked Recessive transmission, AD, and AR
Most common Ectodermal Dysplasia
Hypohidroticprevalence: 1:100,000 births
Incontinentia pigmenti oral manifestations
hypodontiaconical crownsdelayed eruptionpremature teethcleft lip/palate
What is the most common type of diabetes in children?
70% have Type I, Insulin Dependent Diabetes Mellitus 1:400-600 children/adolescents Peak onset: 10-14 y; mini-peak onset: 4-6 y 30% type II
Triad of symptoms for Type I diabetes
- Polydipsia (increase in thirst and appetite) 2. Polyuria (frequent urination) 3. Weight loss*, FTT *polyphagia may not always occur in children
Type I diabetes has what cause
Absence or destruction of beta cells in pancreas leads to absolute deficiency of insulin Causes: genetics (HLA types DR3, DR4) environmental (e.g. Enteroviruses, being breastfed lowers risk), other – developmental (congenital absence), secondary damage (CF), chromosomal (Down, Turner, Kleinfelter, Prader-Willi syndromes) Tx: insulin injections, insulin pump, diet, pancreatic islet cell transplant (experimental)
What is the diagnosis of Type I diabetes?
Symptoms + blood glucose levels measured: fasting glucose is ≥ 120 mg/dL, random ≥ 200 mg/dL HbA1C is elevated when blood glucose is high. Good metabolic control then fewer periodontal implications Normal HbA1C = 7-9
How many teenagers with IDDM have periodontitis?
10 – 15% because of - increased rxn to plaque - increased severity/destruction 3x more perio disease than general population - impaired immune fn (PMN defects) - decreased vascular efficiency
For pts with type I diabetes,What should fasting blood glucose be? Random blood glucose? Normal glycosylated hemoglobin?
Fasting : 120 mg/dL Random: < 200 mg/dL HbA1c (normal is 7-9%)
Type II diabetes is what % of pediatric diabetes?
30% with increasing incidence. Body doesn’t use insulin properly (insulin resistance with relative deficiency) Characterized by hyperglycemia, vascular disease, and neuropathy
What is acanthosis nigricans a symptom of?
Type II diabetes Cutaneous marker of insulin resistance (especially on neck) Leathery or velvety, thickened to papillary brownish patches in flexural sites, axilla, neck, groin, fingers, lips
What are common Type II diabetes medications?
Metformin – reduces glucose production in liver and increases insulin sensitivity (drug of choice for children >10 yrs) oral hypoglycemics injected insulin Diet management and exercise crucial Eat foods wit low to medium Glycemic Indexfat and protein have minimal effect on blood glucose
T/F:Xerostomia is a common oral finding in diabetes.
True increased gingivitis, periodontitis prolonged infxs, candidiasis, xerostomia, sialadenosis delayed wound healing altered sensations: burning, taste odontalgia (microangiopathy) OSA (DM II) Lichen planus, acetone breath, ?benign migratory glossitis (DM I) AAP recommends annual periodontal eval starting at age 6!
T/F: The level of plaque control is more important than level of diabetic control in the severity of the gingival inflammation.
False. The level of diabetic control is more important than plaque control
Dental management of diabetes
AM appts! Short appts Normal insulin dose + normal meal day of appt Ask about hx of hypoglycemia Bring glucometer to appt, check pre-op (if low, consider carbohydrate before starting case) Have source of glucose available Maximize prevention (perio), manage infections aggressively (consider antibiotics), discuss diet/alcohol/tobacco effects on disease control
Diabetes insipidus has what physiologic basis?
ADH deficiencyantidiuretic hormone deficiency
What are symptoms of mild, moderate, and severe hypoglycemia?
Mild: hunger, weakness, sweating, increased pulse, pale moist skin, confusion, sweating Moderate: incoherence, uncooperativeness, belligerence Severe: LOC, tonic/clonic mvmts, hypotension, hypothermia Hypoglycemia management: Administer glucose (tabs, juice, honey, hard candy, frosting); retest blood sugar; repeat in 15 min if not improved. Insulin shock (<40 mg/dL): administer dextrose sublingual, IV or glucagon IM
Two types of GERD are:
- Symptoms and tissue damage: esophagitis, erosive GERD 2. symptoms and no tissue damage: non-erosive GERD
The muscle ring at the distal end of the esophagus is called what?
Lower esophageal sphincter
What is a differential dx for GERD?
Eosinophilic esophagitis – co-exists with atopic conditions like eczema, asthma, rhinitis
What are some oral s/s of GERD?
difficult of painful swallowing gingival inflammation oral erosions and ulcers hypersalivation palatal erythema, petechiae coated or discolored tongue halitosis enamel erosion (mx palatal surfaces first then occlusal surfaces) higher salivary microbial colonization increases risk for caries! Use fluoride varnish, topical f-, calcium phosphate paste for sensitivity; do not brush teeth after vomiting
What are some medications for GERD?
Antacids (neutralize stomach pH) Foaming agents (coat stomach contents) H2 receptor antagonists – inhibit acid production proton pump inhibitors – impede acid production (better drug)
If HIV viral load is undetectable, is the pt still infectious?
Yes. Viral load < sensitivity of test
HIV dental considerations:
Ck labs Neutropenia in 50% children with AIDS, 10% asymptomatic children Anemia: 20-80% Thrombocytopenia infectious diseases common immunologic disorders common oral side effects of HIV drugs (xerostomia, vomiting, taste alterations, sucrose content) over-retained primary teeth, delayed eruption dental caries and enamel hypoplasia symptomatic orofacial lesions referred pain: sinusitis, otitis media, neuropathies increased risk for Vit D deficiency
Presence of oral lesions may signal advancing HIV. T/F
Oral lesions are common in pediatric HIV pts (up to 80%). Oral lesions are associated with decreased immunity and may signal advancing disease CD4 depletion common with presence of oral infections
What is the recommended recall interval for pt with HIV?
Every 3 mo Prevention is critical — Regular recalls, every 3 months — Chlorhexidine may be helpful, if oral hygiene is poor — Fluoride supplementation as indicated Caretaker may be sick and may not be as involved in child’s oral health Poor access to medical and dental care may be complicating factor
Which systemic antiviral agent is approved for pediatric use in HSV infection?
Zovirax (acyclovir) 15 mg/kg 5x/day Famvir and Valtrex are not approved for pediatric use
Hairy leukoplakia is caused by which virus?
EBV lateral border of tongue, white plaques, asymptomatic/burning sensation, lesion waxes and wanes
Viruses associated with cancers in HIV-infected children
EBV, HHV-8, HPV Kaposi’s – HHV-8 lymphoma – EBV, HHV-8
What are the 3 most common pediatric cancers?
- Leukemia 30% leading cause of cancer death <1 yo and 10-19 yo 2. CNS Tumors 17% leading cause of cancer death ages 1-5, most common solid tumor of childhood 3. Neuroblastoma 7% most common malignancy in the first year of life 4. Non-Hodgkin lymphoma and Wilms tumor 6% 5. Hodgkin disease 5% 6. Rhabdomyosarcoma 3% most common soft tissue sarcoma of childhood
What are the most common sinonasal malignancies and what are the s/s?
- Sarcomas esp rhabdomyosarcoma 76% 2. Carcinoma 18% 3. Esthesioneuroblastoma 6% s/s facial swelling and pain, orbital displacement
Does early detection of pediatric cancer influence outcome?
No.
75% of patients will be long-term survivors. There will be late effects of therapy however, like
- problems with organ function, growth & development
- problems with neurocognitive fn and academic achievement
- increased risk for 2nd cancer
- challenges with career opportunities
- access to health care/insurance (should no longer be with ACA!)
T/F Some genetic diseases predispose children for cancer. This includes Down syndrome and multiple endocrine neoplasia, 2B.
T
Neurofibromatosis, type I
AD, 1:3000 births, 50% spontaneous mutation
Stigmata: neurofibromas, café au lait macules, freckling in axillary or inguinal regions, Lisch nodules, CNS disorders
Oral lesions in 70%: enlarged fungiform papilla 50%, oral fibromas 25%, widened mandibular canal 25% and foramen
Tx: excision if cosmetic concern or obstruction
Malignant transformation rate: 5% for large lesions
Some conditions that have café au lait spots:
Neurofibromatosis
McCune-Albright syndrome
(endocrine disease, polyostotic fibrous dysplasia, precocious puberty in F)
Tuberous sclerosis
(hamartomatous skin nodules, seizures, bone lesions, enamel hypoplasia, MR)
Fanconi anemia
What is the most frequent type of leukemia?
ALL >80% of leukemias
leukemias represent 30% of all cancers
5 yr survival 90%
Highest risk in white males
AML is 19% of leukemias
5 yr survival 50%
Diffuse gingival enlargements and chloroma (oral mass) are likely to be seen in (AML/ALL).
AML
ALL: petechiae of skin, mucosa (50%), NUG, cellulitis, persistent ulcers, gingival bleeding and mobile teeth
Kidney (#1) and liver (#2) organ transplants are the most common among children T/F?
T.
Need lifelong immunosuppressive therapy w/any transplant
Kidney transplant indicated w/end stage renal disease.
98% 1 yr, 94% 5 yr survivals
Acute renal failure: blood loss from trauma, dehydration, poisons/meds, disease, necrosis, obstruction of UT
Chronic renal failure: cystic dx, anatomic abnormalities, glomerulonephritis
What are the 2 types of dialysis?
Peritoneal dialysis (dialysate fills abdomen, capillaries move waste into dialysate in abdomen, dialysate is drained/discarded)
Hemodialysis (takes blood out of body and filters it)
What osseous changes are seen with kidney patients?
Renal osteodystrophy
- loss of lamina dura
- demineralized bone, loss of trabeculation (ground glass)
- localized radiolucent lesions (giant cell lesions)
- fracture or tumors from secondary hyperparathyroidism
T/F Erythema of oral mucosa and enlarged pulp chambers are examples of dental findings in renal pts.
F.
- Pallor of oral mucosa, gingival bleeding, increased calculus
- malodor, metallic taste, xerostomia, uremic stomatitis
- Increased salivary pH, reduced caries rate
- Enamel hypoplasia/discoloration, tooth erosion
- delayed tooth eruption, eruption of rootless teeth
- glossitis
- enlarged major salivary glands
- Petechiae and ecchymoses
- soft tissue calcification, PDL widening, pulp obliteration
- renal osteodystrophy - loss of lamina dura, radiolucent giant cell lesions, lytic areas of bone, ground glass appearance (decreased trabeculation), bone demin
- anemia, thrombocytopenia, defective platelet fn
- increased infection
Pediatric liver transplantation could be tx for what 4 main underlying diagnoses?
- Cholestatic liver disease (biliary atresia most common cause)
- Fulminant hepatic disease (drugs, acetaminophen, toxin exposure)
- Metabolic liver disease
- Liver tumors (hepatoblastoma)
T/F Yellow stain in enamel and green discoloration of gingiva, tongue, buccal mucosa and floor of mouth are some dental findings of liver failure.
F. Green stain in enamel (biliverdin)
Yellow discoloration of oral soft tissues (jaundice)
Enamel hypoplasia due to metabolic disturbance
petechiae, ecchymoses
Delayed eruption
Enlarged pulp chambers
fetor hepaticus: sweet, musty breath
mucosal bleeding
What are some important considerations for management of hepatic pts?
- Altered drug metabolism
- Similar considerations to pts receiving chemo
- may be infectious if HBV or HCV is cause
- malnutrition
- frequent recall
- immunosuppression
- bleeding considerations
- frequent recall
T/F Liver transplant is one of the least successful solid organ transplants.
F. It is one of the most successful.
600 performed annually
12,000 total pediatric liver transplants
91% survival 1 yr, 87% 5 yr
T/F:
Chronic periodontitis may contribute to organ transplant rejection.
True.
IL-6 can identify individuals at risk for organ rejection (higher IL-6 w/ chronic perio)
Other dental mgt considerations of solid organ transplant:
risk for gingival overgrowth – specific to this group
reinforce oral prevention program
monitor and treat opportunistic infections
caution w/ antibiotics – nephrotoxicity
manage xerostomia
risk of “de novo” dysplasias and malignancies (epithelial dysplasia, NHL, SCC, BCC)
What type of side effects does immunosuppressive therapy have on pts?
- Increased risk for infection
- Change in appearance
- Cushingoid appearance
- acne
- obesity
- hirsutism
- increased cancer risk
- hypertension
- osteoporosis
Name a few immunosuppressant drugs.
Cyclosporine
- use in peds pts decreased to <15% in ’96)
- gingival enlargement in 25%
Tacrolimus
- candidiasis, ulcers
Azathioprine
- thrombocytopenia, leukopenia, anemia, skin cancer
Steroids
- insomnia, nervousness, infections
Calcium Channel Blocker: Nifedipine
- may be rx’d to minimize nephrotoxic/HTN effects of cyclosporine and tacrolimus
- gingival overgrowth
Mycophenolate Mofetil (MMF) - leukopenia, GI problems, myelosuppression
Sirolimus & Everolimus
- delayed wound healing, bone marrow suppression, oral ulcers
What are 3 symptoms of OSAS Obstructive Sleep Apnea Syndrome?
- Nightly snoring
- Disturbed sleep
- Daytime neurobehavioral problems
T/F There is a cure for muscular dystrophy
F. No cure or effective treatment
Pseudohypertrophic MD (Duchenne) = most common
neuromuscular disease of childhood
X-linked recessive (all males affected)
By what age are pts typically unable to walk? What is the life expectancy?
Can’t walk by 12.
Universally fatal – death prior to age 30 from respiratory or cardiac complications
25% demonstrate MR
elevation of creatine kinase by 50-100 times related to muscle damage
What is Spina Bifida?
Neural tube defect
Etiology can be:
- folic acid deficiency
- maternal insulin-dependent diabetes
- maternal obesity, hyperthermia, antiepileptic drugs
1,500 infants/yr in US
What are 3 classifications of spina bifida?
- Latex allergy precautions
- seizures, obesity
- Decreased bone density
- MR?
- fear of choking w/reclining – consider treatment inclined slightly and more upright as supine position may be difficult
- abx px for VV or VA shunt?
What are some symptoms of hydrocephalus and how common is it?
Incidence: 1 in 500 children
Symptoms: rapid increase in head circumference in infants, vomiting, seizures, downward casting of eyes (“sunsetting”), sleepiness, irritability, blurred vision, inability to balance
T/F Decreased salivary bacteria is a common oral finding of pts with G-tube.
F. Increased salivary bacteria
- aspiration pneumonia
What are some major complications of bulimia?
Suicide, depression, substance abuse, cardiac arrythmias & arrest, esophagitis/pancreatitis, seizures, aspiration, impaired renal function.
What is a common salivary gland finding with pts with bulimia or anorexia nervosa?
10-15% have unilateral or bilateral parotid gland enlargement and dry mouth
What is Russell’s sign?
Calluses on the knuckles or back of the hand due to repeated self-induced vomiting (from knuckles contacting incisors)
What is the most common eating disorder in the US?
Binge eating disorder
recurrent episodes of binge eating in the absence of the regular use of inappropriate compensatory behaviors such as purging, fasting, excessive exercise
What are the most common treatments for depression?
SSRI (selective serotonin reuptake inhibitors)
- cognitive behavior therapy
- interpersonal therapy for adolescents
T/F Depression has a high risk of recurrence.
T.
40% within 2 years
72% within 5 years
What are some common meds for tx of depression?
ADHD meds (stimulants or non-stimulants)
Meds for depression, phobias, anxiety, eating disorders, OCD, personality disorder, PTDS like
- SSRI (fluoxetine, paroxetine, sertraline, fluvoxamine, citalopram)
- tricyclic antidepressants (second choice after SSRIs)
(Imipramine, nortriptyline, desipramine, amitriptyline)
*xerostomia
Neuroectodermal tumors of infancy are
a. Smooth surfaced, expansile lesions
b. More common in the mandible
c. Most likely to develop at 9-12 months
d. Vascular
e. Well circumscribed radiolucencies
a. Smooth surfaced, expansile lesions
b. More common in the maxilla
c. Most likely to be present at birth
d. Avascular
e. Poorly circumscribed radiolucencies “floating teeth”
Melanotic neuroectodermal tumor of infancy
Sturge-Weber syndrome is NOT associated with
a. facial angiomatosis
b. Hearing loss
c. Hemiplegia
d. Mental retardation
e. seizures
b. Hearing loss
Sturge-Weber:
- Vascular malformations of face and brain
- Congenital port-wine stain follows trigeminal nerve
- Ipsilateral facial angiomatosis
- There is ocular involvement, MR, hemiplegia (CNV involved), seizures
- Oral: hypervascularity, pyogenic granulomas, gingival overgrowth, alveolar bone loss, bleeding; supernumerary teeth; overgrowth of bony maxilla, enamel hypoplasia, accelerated eruption, talon cusp
What is histiocytosis?
Dendritic cell and macrophage
(White blood cells) proliferation and infiltration into tissues
The three types of Langerhans Cell Histiocytosis
- Unifocal – (formerly known as Eosinophilic Granuloma misnomer) no extraskeletal involvement – excellent prognosis
- Multifocal – mostly seen in children age 2-10, fever, bone lesions, diffuse eruptions. Good prognosis.
Hand-Schuller-Christian triad - diabetes insipidus,
- exophthalmos, and
- lytic bone lesions
- Multifocal multisystem – aka Letterer-Siwe disease. Children ≤ 2 yo, poorest prognosis
LCH has pathognomonic radiographic presentation of what?
“floating teeth”*** caused by bone lesions.
Punched out lesions of skull
10% show oral involvement
(which is the usual initial infiltration area)
“floating teeth” can also be seen in melanotic neuroectodermal tumor of infancy, traumatic bone cyst.
What is a differential of eczema?
- neoplasm
- immunodeficiencies
- metabolic defects
Criteria: pruritus, pattern of skin involvement (young children – extensor surfaces, cheeks, forehead, neck; older children – flexor surfaces), family hx, young age of onset, elevated serum IgE and eosinophil counts
Eczema is aka and mostly presents before what age?
Atopic dermatitis
90% present by age 5
Eczema can be exacerbated by what in 1/3 of cases?
Food allergen
Dental considerations for eczema
- High dose corticosteroids – consult pmd
Urticaria is also known as what?
Hives.
Definition: superficial dermal layers affected by pruritic, erythematous raised lesions (blanch with pressure)
Angioedema
DEEP swelling that affects the face, extremities, genitalia, occasionally tongue and larynx (under the skin, not on surface)
Urticaria and angioedema are associated what percent of the time?
40% hives with underlying swelling
Hereditary Angioedema has what symptoms?
Submucous or subcutaneous edema
- non-pitting
- tensely swollen
- can be painful?
- NOT erythematous/warm/pruritic
Autosomal dominant
deficiency in functional C1 esterase inhibitor (complement system)
What can trigger hereditary angioedema?
Trauma
medical/dental procedure
stress
menstruation
infections
oral contraceptives
medications
foods, plants
heat, cold, sun, pressure, exercise
What areas are affected by hereditary angioedema?
- Lips
- Eyelids
- Tongue
- Extremities
- Genitalia
- Oropharyngeal/laryngeal is most concerning (bc of airway obstruction potential)
Is Epi/antihistamines tx for acute attack of hereditary angioedema?
NO.
Acute attack: Purified C1-esterase inhibitor (Berinert), recombinant protein inhibitor of kallikerin, ecallantide(Kalbitor)
Epi and antihistamines are not useful
Flaitz lecture: could use benadryl for mild cases; severe cases – IM Epi, IV steroids and antihistamines; C1-INH deficiency – Danazol, stanozolol for prevention
Food allergy is mediated by what Ig and what is its overall prevalence?
IgE-mediated in 6-8% of kids < 3 yo.
2% prevalence overall
What are the big 8 food allergens?
- Eggs
2. Peanuts - cow’s milk
- Soy
5. Tree nuts
**6. Fish - shellfish**
- wheat
ones in bold persist after childhood
Latex allergy prevalence is?
5–10% of general population
higher (up to 17% of health care workers)
Irritant contact dermatitis to latex is an immune-mediated reaction, T/F?
F. Caused by glove wearing/handwashing/sanitizers/other chemicals
Non-immunological mediated
dry/itchy/irritated skin
Allergic contact dermatitis latex allergy is a delayed hypersensitivity reaction T/F?
Yes. It is t-cell mediated
rash/redness/itching 24-48 hrs after contact
Immediate allergic reaction to latex frequently leads to anaphylaxis T/F?
No – rarely anaphylaxis.
It can progress to urticaria, allergic rhinitis, sneezing, wheezing, coughing, difficulty breathing.
IgE-mediated
swelling, redness, itching
Who is at risk for latex allergy?
- Ppl who have undergone multiple surgical procedures
- spina bifida 18-73%
- first surgery before age 1
- spinal cord trauma, urogenital/GI malformations, neurogenic bladder, hydrocephalus w/ VP shunts
- atopic individuals
- occupational exposure
- food allergies like avocados, bananas, chestnuts, figs,kiwis, turnips, tomatoes, potatoes
- fruit allergy 11% risk of concurrent latex allergy
Name 3 rheumatologic diseases
1. Juvenile (Idiopathic) Arthritis
- consider that person is in pain and may have TMD
2. Vasculitides
- Wegener granulomatosis (involves medium and small arteries, venules, arterioles, extremely rare in children)
- Bechet (very rare in children, oral apthous ulcers, ocular disease, large vessel vasculitis 33%, small vessel common)
3. Systemic Lupus Erythematosus
- most common presentation in childhood: fever, malaise,
failure to thrive
- malar rash only present in 1/3 patients
- Assess kidney function
- increased susceptibility to infection
- depletion of antibodies
- secondary heart damage
- Sjogren syndrome can be secondary complication
Chondroectodermal dysplasia aka
Ellis van creveld
“6 fingered dwarfism” – short distal extremities, short stature, polydactyly
40-50% have cardiac defects
chondrodysplasia
hidrotic ectodermal dysplasia
neonatal teeth, multiple frenae
hypodontia, enamel hypoplasia, microdontia
may have delayed exfoliation/eruption or accelerated eruption BOTH! *
*Other condition as having both delayed exfoliation and accelerated eruption is OI.
Paramedian lip pits are often associated with what syndrome?
Van der Woude
Paramedian lip pits:
persistence of lateral sulci on embryonic arch
congenital invaginations of lower lip
Heck’s disease is caused by what and aka?
HPV 13, 32
=Multifocal Epithelial Hyperplasia
proliferation of oral squamous epithelium
seen in certain ethnic groups (Mexicans, Navajo, South Americans), seen in children
characterized by multiple raised papules on lower lip, buccal mucosa, tongue
Which syndrome?
- Oculo-auriculo-vertebral spectrum syndrome
- Anomaly of 1st and 2nd branchial arch
- Unilateral microtia, macrostomia
- Failure of ramus & condyle formation
- 50% have cardiac pathology: VSD, PDA
- colobomas
- skeletal involvement
Goldenhaar syndrome
aka
Hemifacial Microsomia
What % of hemifacial microsomia patients have cardiac pathology?
50%
etiology unknown
unilateral microtia, macrostomia, failure of formation of mandibular ramus and condyle
1st and 2nd arch
Tx: distraction osteogenesis, orthognathic surgery
Depressed nasal bridge, trident hands, enlarged head and autosomal dominant inheritance are features of
- Achondroplasia
- Chondroectodermal dysplasia
- Hallerman-Streiff syndrome
- Hypopituitarism
- Morquio syndrome
Answer: A. Achondroplasia- short limb dwarfism, caudal narrowing of spinal canal, 1 in 20,000 (most common form of dwarfism), hypotonia (caution sedation), trident hands= short metacarpals & phalanges, midface hypoplasia, frontal bossing, hypodontia, delayed primary exfoliation/perm. Eruption
Chondroectodermal dysplasia (Ellis-van Creveld)- hypodontia, short distal extremeties, polydactyly, nail hypoplasia, autosomal recessive, neonatal teeth, enamel hypoplasia, conical crowns, microdontia, multiple frena, 50% have cardiac defect usu ASD, prenatal onset of short stature (form of dwarfism), seen in Amish populations
Hallerman-Streiff syndrome- hypodontia or hyperdontia, mandibular hypoplasia, high palatal vault, premature/natal teeth, delayed primary exfoliation, malar hypoplasia, microphthalmia, small pinched nose, hypotrichosis (less than normal amount of hair), “wormian” bones (abnormal intrasutural bones), narrow upper airway
Hypopituitarism- growth hormone deficiency, small, well-proportioned unlike achondroplastic dwarf; dental findings- hypodontia, delayed tooth eruption, smaller mandible, microdontia, shortened roots, crowding/malocclusion due to small dental arches, hypofunction of salivary glands, higher caries/perio risk
Morquio syndrome- inherited disease of metabolism in which body cannot break down glycosaminoglycans (formerly called mucopolysaccharides); autosomal recessive, abnormal bone development including spine, heart problems, loss of nerve function below the neck; normal cognitively
Which syndrome?
Prenatal onset of growth alteration
microcephaly
short palpebral fissures
decreased IQ
smooth upper lip (indistinct philtrum)
ptosis
Fetal alcohol syndrome
The condition associated with dentin abnormalities is
- Apert syndrome
- Cleidocranial dysplasia
- Down syndrome
- Ehlers-Danlos syndrome
- Gorlin syndrome
Answer: D. Ehlers-Danlos syndrome (collagen defects)
Other conditions with dentin abnormalities:
Vit D resistant rickets,
hypoparathyroidism,
pseudohypoparathyroidism,
Albright’s hereditary osteodystrophy
Apert syndrome- supernumerary teeth
Cleidocranial dysplasia- supernumerary teeth
Down syndrome- supernumerary teeth, taurodontism
Gorlin-
Vitamin D-resistant Rickets is also called ________ rickets and is what inheritance pattern?
Hypophosphatemic rickets
X-linked dominant; 1:20k births
Mutation in zinc metalloproteinase gene
- decreased resorption of phosphate in renal tubules
- short bowed extremities
- dental: large pulps, high pulpal horns, hypomindentin, abscesses, enamel may be spared
Tx: calcitriol, phosphate
Ehlers-Danlos syndrome
AD for classic forms (80%) - Flaitz;
handbook says x-linked
Stigmata: hyperelastic skin, scarring, hypermobility of joints, subluxation of TMJ, bruise easily delayed healing
Oral: periodontitis, Gorlin sign, bleeding, pulp stones, +/- short roots, prominent cusp tips, hypoplastic enamel, dentin abnormalities
Tricho-dento-osseous syndrome has what dental characteristics?
- Pitted teeth
- Enamel hypomineralization
- Taurodontism (w/ PARLs)
- delayed eruption
- dental hypoplasia
- sclerotic dense bone
- dolicocephaly with frontal bossing
(AI + taurodontism + nail and hair defects)
Syndromes associated with supernumerary teeth
Aperts
Cleidocranial dysplasia
Gardner
Crouzon*
Sturge-weber
Orofacialdigital syndrome*
Hallerman-Strieff*
cleft lip/palate*
Down syndrome
*also hypodontia
Syndromes associated with hypodontia
Seckel
Williams
Rieger
achondroplasia
chondroectodermal dysplasia
ectodermal dysplasia
Crouzon*
cleft lip/palate*
incontinentia pigmenti
orodigitalfacial syndrome*
Hallerman-Strieff*
*also hyperdontia
Conditions with microdontia
Crouzon syndrome
Down syndrome
chondroectodermal dysplasia
ectodermal dysplasia
hemifacial microsomia
oligodontia
Cleidocranial dysplasia oral manifestations
Maxillary hypoplasia
+/- cleft palate
small maxillary sinuses
supernumerary teeth
delayed dental development and eruption/delayed primary exfoliation/failure of permanent tooth to erupt
enamel hypoplasia
deficient cellular cementum
brachycephaly, frontal and parietal bossing, depressed nasal bridge, Wormian bones
CBFA1 (RUNX2) gene defect
1:100k prevalence
inherited bone disorder, AD w/ 40% spontaneous mutation
affects craniofacial, clavicles, dental
Tx: multidisciplinary care
Oro-facial-digital syndrome
Type 1: most common
X-linked dominant
syndactyly, clinodactyly, extra digit
intellectual disability
hypoplastic alar cartilage
hypotrichosis
Oral anomalies:
- hamartoma/choristoma on tongue
- multiple frenae
- bifid, multilobed tongue
- hypodontia (lower 2s) or hyperdontia (upper 3s)
- cleft of palate, alveolus
- “bumpy jaw”
- brachycephaly
This syndrome is associated with self-mutilating behavior, MR, spastic CP, choreoathetosis.
Lesch-Nyhan Syndrome
X-linked recessive
absence of hypoxanthine-guanine
Which of the following condition : dental anomaly associations is correct?
- Crouzon syndrome : taurodontism
- Hallerman-Streif : microdontia
- Ichthyosis : accelerated eruption
- Pseudohypoparathyroidism : microdontia
- Sturge-Weber syndrome : dens evaginatus
Answer: E. Sturge-Weber and dens evaginatus- talon cusp (lobodontia), also supernumerary teeth;
other syndromes with talon cusp- Mohr, Rubinstein-Taybi, orofacial digital II syndrome
Crouzon- hyper/hypodontia, inverted V-shaped palate, V-shaped arch, maxillary hypoplasia, brachy, microdontia or macrodontia, exopthalmia, no hand anomalies, hypertelorism
Hallerman-Streif- hyper/hypodontia, delayed exfoliation;
Ichthyosis- delayed primary exfoliation, ankylosis, dilaceration;
Pseudohypoparathyroidsim- dentin abnormalities (e.g. dentin dysplasia), enlarged pulp, small crowns, short blunted roots, pitted enamel, enamel hypoplasia, enamel attrition, delayed eruption, malformed or impacted teeth, partial anodontia
The syndrome that presents with natal teeth 25% of the time is
- Chediak-Higashi
- Ellis-van Creveld
- Letterer-Siwe
- Han-Schuller-Christian
- Williams
Answer: B. Ellis-van Creveld; also, pachyonychia congenita
Other syndromes with natal teeth are Histiocytosis X and Pfeiffer
Seckel syndrome
Hypodontia
growth deficiency beginning at birth
microcephaly, facial hypoplasia
MR
AR
What tests extrinsic clotting factors and is increased in patients with liver disease?
- Bleeding time
- International normalized ratio
- Partial thromboplastin time
- Platelets
- Prothrombin time
Answer: Prothrombin time (PT)
Pneumonic- PET (extrinsic, PT) and PITT (intrinsic PTT)
Gardner syndrome presents with supernumerary teeth and what other oral manifestations?
Supernumerary teeth
delayed eruption
multiple osteomas of the jaw
also:
epidermoid and dermoid cysts
polyposis large intestines w/ high malignant potential
AD
The condition featuring malar and mandibular hypoplasia, malformed ears with associated hearing loss, abnormalities of the eye and airway anomalies is
- Apert syndrome
- Cleidocranial dysplasia
- Crouzon syndrome
- Pierre Robin sequence
- Treacher Collins syndrome
Answer: Treacher Collins syndrome
malar and mandibular hypoplasia, downsloping palpebral fissures, malformed ears with associated hearing loss, and abnormalities of the eye including lower lid coloboma and absent lashes, cleft palate 30%. Airway anomalies may occur, causing life-threatening problems in the newborn period.
Intelligence is generally normal
Gene: TCOF1
increase with paternal age
defect of 1st and 2nd branchial arches
Tx: distraction osteogenesis
What syndrome?
Craniosynostosis (tower skull), mental disability, syndactyly
Apert Syndrome
= acrocephalosyndactyly
AD, increase with paternal age
ocular proptosis, hypertelorism, parrot nose
midface hypoplasia;
narrow, high palate;
cleft palate 75% or bifid uvula;
delayed or ectopic eruption;
supernumerary teeth;
shovel-shaped incisors;
V-shaped maxilla; class III with AOB; crowded dentition
Tx: craniofacial surgery
ADHD
- Has a genetic predisposition
- Is considered a developmental disorder
- Leads to decreased caries rates
- Starts before age 4
- Treatment is most effective with depression medications
Answer: has a genetic predisposition; neurobehavioral disorder, increased caries risk, symptoms present prior to age 7, treated with stimulant medications
Spina bifida can be caused by all of the following except
- Folic acid deficiency
- Maternal obesity
- Maternal IDDM
- Paternal age
Answer: Paternal age; folic acid deficiency most common, also maternal epilepsy medication use
A portion of the cerebellum protruding through foramen magnum into the spinal canal that causes headaches is a/an
- Arnold-Chiari malformation
- Ebstein anomaly
- Hydrocephaly
- Meningocele
- Spina bifida occulta
Arnold-Chiari malformation
*for patients with this, consider tx in a more upright position (pts may feel discomfort and/or choking on reclining)
The syndrome also known as acrocephalosyndactyly, which features irregular craniosynostosis, midface hypoplasia, syndactyly, cognitive delays and is associated with increased paternal age is
- Cleidocranial dysostosis
- Crouzon syndrome
- Apert syndrome
- Gardner syndrome
- Orofacialdigital syndrome
Apert Syndrome
The most common pediatric (0-15years) cancer by type is
- Wilms tumor
- Non-Hodgkin lymphoma
- Leukemia
- CNS tumors
- Neuroblastoma
Answer: c. Leukemia (30%),
CNS 17%,
Neuroblastoma 7%,
Non-Hodgkin lymphoma 6%,
Wilms tumor 6%
How many days does a normal red blood cell survive?
2 days
12 days
30 days
120 days
Answer: d. 120 days;
sickled RBC survives 12 days
Which of the following would lead you to defer elective dental treatment
- ANC = 1000
- Platelets = 35,000
- Red blood cells = 4million/mm3
- Lymphocytes 25%
Answer: B. Platelets 35,000 (normal 150-340K),
ANC >1000 ok,
RBC 4-6 mil normal,
lymph 25-30% normal
What is the most common comorbid medical condition in persons who have developmental disabilities?
Seizure disorder
Which syndrome?
6th and 7th nerve palsy
hypoplasia of central brain
micrognathia
myopathy
Moebius syndrome
☹ baby
CHARGE association
Coloboma of the eye
Heart anomaly (TOF)
Atresia, choanal
Retardation (growth/cognition)
Genital anomalies
Ear anomalies
VATER(s)
Vertebral defects
Anal atresia
Tracheo-esophageal fistula
Radial and renal dysplasia
Single umbilical artery
Blepharophimosis syndrome
Narrowing of eye opening (blepharophimosis)
lateral displacement of inner canthi
ptosis (droopy eyelids)
cupping of ears
hypotonia
premature ovarian failure in type I
AD
normal intelligence
Pierre Robin Sequence
Triad of:
micrognathia/mandibular deficiency
glossoptosis
cleft palate (U-shaped)
15-25% w/ heart disease
Fraser Syndrome
Aka Cryptophthalmos Syndrome
cryptophthalmos (fusion of eyelids)
defect of auricle
cleft palate
mental deficiency 50%
renal defects
laryngeal stenosis
partial syndactyly
20% still born, 20% die w/I 1st year
AUTOSOMAL RECESSIVE
Branchio-Oto-Renal syndrome
Aka Melnick-Fraser Syndrome
branchial arch II anomalies – branchial cleft cysts
hearing loss, preauricular pits/tags, malformed pinnae
renal abnormalities
long, narrow face; constricted palate; deep bite
1:40,000
AD
Which syndrome?
Branchial arch abnormalities – arches I and II
abnormal skin patches/hemangiomas
Ocular abnormalities
microphthalmia, anophthalmia, coloboma, nasolacrimal duct stenosis
Facial abnormalities
cleft lip/palate, pseudoclefts, hypertelorism, upslanting palpebral fissures, broad nose, facial mm. weakness, malformed ears, premature graying of hair
Branchio-oculo-facial syndrome
“Jay Leno” syndrome
Waardenburg Syndrome
congenital hearing loss
changes in pigmentation of hair, skin eyes (e.g. different colored eyes, pale blue eyes, white forelock of hair, premature graying)
broad mandible
AD
Wildervanck Syndrome
Aka Cervico-oculo-acoustic syndrome
asymmetry with short neck, low hairline
fusion of two or more cervical vertebrae (Klippel-feilanomaly)
abnormal eye movements – Duane anomaly (limitation of horizontal eye movement/abducens paralysis)
congenital hearing impairment, preauricular skin tags/pits
normal intelligence
affects females primarily
Mitochondrial disease
Multi-organ disorder results in cell injury
affects 1:4000 children
increases susceptibility to MH
T/F:
Mucopolysaccharidosis syndromes are mostly AD inheritance pattern.
False – most are AR
Hurler, Scheie, Sanfilippo-A,B, Morquio-A,B and Maroteaux-Lamy are AR; Hunter is X-linked recessive
Oral findings: macroglossia, gingival hyperplasia (GAGs in gingival tissue), tooth spacing, enlarged dental follicle, unerupted teeth (delayed eruption), enamel hypoplasia
What are the significant complications of Multiple Endocrine Neoplasia 2B?
Medullary carcinoma of thyroid, pheochromocytomas (neuroendocrine tumor of the medulla of the adrenal gland) – hypertensive crisis
Marfanoid body, narrow face, full lips, mucosal neuromas
Oral findings: multiple, soft papules or nodules; pink; smooth surface; nontender
Site: eyes, lips, tongue, buccal mucosa, gingiva
Tx: excision of large mucosa neuromas
AD
Which syndrome?
Macroglossia
Sleep apnea
Omphalocele, umbilical hernia
Cytomegaly of the adrenal cortex
Renal anomalies
Postnatal somatic gigantism
Severe hypoglycemia
Neoplasms (nephroblastoma – most common)
Beckwith-Wiedemann Syndrome
Which syndrome?
Hypotonia
obesity
small hands, feet
eat “odd” things
chromosome 15 deletion, inherited from father
Prader-Willi syndrome
Chromosome 15 deletion inherited from mother is Angelman syndrome (“puppet-like” gait, bouts of laughter, open-mouth facies, seizures, MR)
